CNS Pathoma Flashcards
Neural tube defects are associated with
Low folate levels prior to conception
How can neural tube defects can be detected?
Elevated AFP in amniotic fluid and maternal blood
If a failure to close happens at the cranial end we call it
Anencephaly
Anencephaly=
Absence of skull and brain
Anencephaly results in maternal ________
Polyhydramnios
Description of Anencephaly gross pathology
Frog like appearance
Failure of posterior vertebral arch to close is called
Spina bifida
Spina bifida leads to
Meningocele
Meningomyeleocele
Congenital stenosis of the channel that drains CSF from 3rd to 4th ventricle is called
Cerebral aqueduct stenosis
Cerebral aqueduct stenosis leads to
Accumulation of CSF in the ventricles
Cerebral aqueduct stenosis presents with
Enlarging head circumference
Congenital failure of the cerebellar vermis to develop is called
Dandy-Walker malformation
Dandy-Walker malformation presents with
Massively dilated 4th ventricle with absent of cerebellum
Congenital extension of cerebellar tonsils through foramen magnum
Arnold-Chiari malformation
CNS developmental anomalies list
Anencephaly Spina bifida Cerebral aqueduct stenosis Dnady-Walker malformation Arnold-Chiari malformation type II
Spinal cord lesions list
Syringomyelia Poliomyelitis Werding-Hoffman disease Amyotrophic Lateral Sclerosis (ALS) Friedreich Ataxia
Cystic degeneration of the spinal cord
Syringomyelia
Syringomyelia arises with
Trauma
Arnold-Chiari malformation
Syringomyelia presents with
Sensory loss of pain and temparture
Syringomyelia can cause _______ syndrome
Horner
Horner syndrome =
Ptosis
Miosis
Anhidrosis
Horner syndrome will happen due to
Disruption of the lateral horn of the hypothalamospinal tract
Poliovirus will cause
Poliomyelitis
Poliomyelitis =
Damage to anterior motor horn
Poliomyelitis presents with
Lower motor neuron signs
Main difference btw ALS and syringomyelia
ALS=
Syringomyelia will show lack of sensory impairment
Amyotropic Lateral Sclerosis
Most cases of ALS are ______
Sporadic
Familial cases of ALS will have
Zinc-Copper superoxide dismutase mutation
Degenerative disorder of the cerebellum and spinal cord is called
Friedreich ataxia
Meningitis =
Inflammation of the leptomeningis
Leptomeningis=
Pia and Arachnoid
Meningitis is most commonly due to
Infectious agent
Neonate meningitis causes
GBS
E.Coli
L. Monosytogenes
Children meningitis causes
N. Meningitdis
Adults meningitis causes
Sterp. Pneumo
Most common viral cause of meningitis
Coxsackivirus
Meningitis presents as
Headache
Neck stiffness
Fever
Bacterial meningitis CSF features
Neutrophils
Low glucose
Viral meningitis CSF features
Lymphocytes
Normal glucose
Fungal meningitis CSF features
Lymphocytes
Normal glucose
Complications of meningitis are seen in _________ meningitis
Bacterial
What can be the cause of Global cerebral ischemia?
Low perfusion
Shock
Anemia
Repeated episodes of hypoglycemia
Regional ischemia that results in neurologic deficit for > 24h is called
Ischemia stroke
Regional ischemia that results in neurologic deficit for < 24h is called
Transient Ischemic Attack TIA
Subtypes of Ischemic stroke
Thrombotic
Embolic
Lacunar
Thrombotic stroke results in ______ infarct at the _______
Pale
At the periphery of the cortex
Embolic stroke results in a ______ infarct at the ________
Hemorrhagic
At the periphery of the cortex
Lacunar stroke involves which blood vessels
Small vessels of the brain
In Lacunar stroke, the small vessels of the brain go through ___________ change
Hyaline arteriolosclerosis
Lacunar stroke most commonly involves ______ vessels
Lenticulostriate
Ischemic stroke leads to _______ necrosis
Liquefactive
Earliest change in ischemic stroke
Red neurons
1 month post ischemic stroke gross pathology will look like
Fluid filled cystic space surrounded by gliosis
Bleeding into the brain is called
Intracerebral hemorrhage
Intracerebral hemorrhage is usually due to
Rupture of Charcot-Bouchard microaneurysms
Most common site of intracerebral hemorrhage
Basal ganglia
Bleeding into the subarachnoid space is called
Subarachnoid hemorrhage
Subarachnoid hemorrhage presents as
Sudden headache with nuchal regidity
LP of subarachnoid hemorrhage will show
Xanthochromia (Yellow hue due to bilirubin breakdown)
Subarachnoid hemorrhage happens most frequently due to
Rupture of Berry aneurysm
Most frequent location of Berry aneurysm
Anterior circle of Willis (Branch point of anterior communicating artery)
Berry aneurysm is associated with what diseases
ADPKD
Marfan syndrome
CT scan of Epidural hematoma
Lens shaped lesion
Epidural hematoma usually happens due to
Fraction of the temporal bone with rupture of the middle meningeal artery
Subdural hematoma is usually due to
Tearing of bridging veins between Dura and Arachnoid
Types of brain herniation
Tonsillar
Subfalcine
Uncal
Tonsillar herniation can cause
Compression of brain stem -> Cardiopulmonary arrest
PNS myelination
Schwann sells
CNS myelination
Oligodendrocytes
Inherited mutations in enzymes necessary for production or maintenance of myelin
Leukodystrophies
Multiple sclerosis is caused by
Autoimmune destruction of CNS myelin and oligodendrocytes
Multiple sclerosis is associated with
HLA-DR2
Clinical featured of Multiple Sclerosis
Optic nerve
Blurred vision in one eye
Clinical featured of Multiple Sclerosis
Brainstem
Vertigo and scanning speech
Diagnosis of MS
MRI shows plaques
LP shows increased lymphocytes
Treatment of acute attack of MS
High dose steroids
IFN-B
Progressive mpMultifocal Leuko-Encephalitis is due to
JC virus infection of oligodendrocytes
Central demyelination of pons is called
Central pontine myelinolysis
Central pontine myelinolysis classically presents as
Locked in syndrome
Central pontine myelinolysis is due to
Rapid IV correction of hyponatremia
CNS metastatic lesion usually presents as
Multiple, well circumscribed lesions
3 most common cancer that like to metastasize to the brain
Lung
Breast
Kidney
Most abundant glial cell
Astrocytes
Astrocytes make up the
BBB
CNS tumors in adults usually occurs ________ the tentorium
Above
What is the most common malignant tumor in adult
CNS
Glioblastoma Multiforme
Glioblastoma Multiforme is a tumor of
Astrocytes
Special feature of Glioblastoma Multiforme
Crosses the corpus callosum (Butterfly lesion)
Which tumor produces butterfly lesion
Glioblastoma Multiforme
Histology of Glioblastoma Multiforme
A lot of necrosis
Endothelial cell proliferation
Benign tumor of the Arachnoid cells
Meningioma
What is the most common benign CNS tumor in adults
Meningioma
Meningioma expresses ________ receptor
Estrogen
Meningioma histology picture
Whorled pattern
Psammoma bodies
Schwannoma involves especially which CN?
8th at the CPA (Cerebellar Pontine Angle)
Schwanomma is __ positive
S100
Bilateral Schwanomma is seen in
NF2
Degeneration of cortex leads to
Dementia
Degeneration of brainstem and basal ganglia leads to
Movement disorders
Dementia=
Memory loss
Cognitive dysfunction
No loss of consciousness
Early onset AD is seen in
Familial cases
Down syndrome
Familial cases of AD
What mutation?
Presenilin 1 / 2
Why Down syndrome patient are prone to AD?
Amyloid Precursor Protein is found on chr. 21
__ allele of _____ is associated with increased risk of AD
E4
Apo.E
___ is associated with decreased risk to AD
E2 allele of Apo.E
Morphological changes in AD
Cerebral atrophy
Neuritic plaques
Neurofibrillary tangles
Loss of cholinergic neurons in the nucleus basalis of Meynert
What are neuritic plaque?
Extracellular core compromised of AB amyloid with entangled neuritic processes
What is neurofibrillary tangle?
Intracellular aggregates of fibers composed of hyperphosphorylated tau protein
What is Tau protein
Microtubule associated protein
Multifocal infarction and injury due to HTN/Atherosclerosis/Vasculitis can cause
Vascular dementia
Pick disease is a degenerative disease of
Frontal and temporal cortex
Pick disease is characterized by
Round aggregates of tau protein in neurons of the cortex
Pick disease is characterized by what symptoms
Behavioral and language
Parkinson disease=
Degenerative loss of Dopaminergic neurons in the substantia nigra of the basal ganglia
Basal ganglia helps to regulate ________
Movement
Clinical features of Parkinson disease (4)
TRAP
Tremor
Rigidity
Akinesia
Postural instability
Histology of Parkinson disease
Loss of pigmented neurons in sustantia nigra
Round, eosinophilic inclusions of a-synuclein (Lewy body)
______ is a common feature of late Parkinson
Dementia
Describe Huntington disease
Degeneration of GABAergic neuron in the caudate nucleus of the basal ganglia
What is the genetics background of Huntington
AD
Chr. 4
Expanded CAG repeats in the Huntington gene
Further expansion of repeats during spermatogenesis leads to _________ in Huntington
Anticipation
Rapid movements=
Chorea
Degenerative disease due to prion protein=
Spongiform encephalopathy
Prion protein is normally expressed in
CNS neurons in an a-helical configuration
Spongiform encephalopathy arises with conversion to a
Beta pleated conformation of the prion protein